Paediatrics (RCH) - Research Publications

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    Stability of combined Le Fort I maxillary advancement and mandibular reduction.
    Arpornmaeklong, P ; Shand, JM ; Heggie, AA (Walter de Gruyter GmbH, 2003-11)
    BACKGROUND: There have been reports that correction of severe Class III abnormality by single jaw surgery may invite relapse in the long-term. The purpose of this study was to retrospectively evaluate the stability of combined Le Fort I maxillary advancement and bilateral sagittal split osteotomies for mandibular reduction. METHODS: Thirty patients with a skeletal Class III malocclusion underwent bimaxillary surgery using rigid fixation and interpositional bone grafting of the maxilla. The average age was 24.4 years, and the mean follow-up period was 20 months (Range: 12-63 months). Post-operative changes were measured on lateral cephalometric radiographs using an anatomical best-fit technique. RESULTS: The maxilla was advanced, on average, 6.1 mm (SD: 1.8 mm) and repositioned superiorly at PNS 1.9 mm (SD: 2.1 mm). The mandible was repositioned posteriorly 5.6 mm ISD: 4.2 mm) at menton, which also auto-rotated superiorly. At follow-up, the maxilla relapsed horizontally 0.6 mm (SD: 1.1 mm, p < 0.01) with no significant vertical change. The maxillary central incisors were proclined and the interincisal angle was reduced. Menton relapsed anteriorly 1.4 mm (SD: 2.7 mm, p < 0.01), and gonion rotated superiorly 1.5 mm (SD: 2.3 mm, p < 0.001). In 67 per cent of cases menton moved anteriorly less than 2.5 mm. The overjet and overbite did not change significantly. CONCLUSIONS: The data show that 12-months post-operatively, maxillary advancement combined with mandibular setback was relatively stable in the horizontal and vertical planes.
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    Normal skeletal development of mice lacking matrilin 1:: Redundant function of matrilins in cartilage?
    Aszódi, A ; Bateman, JF ; Hirsch, E ; Baranyi, M ; Hunziker, EB ; Hauser, N ; Bösze, Z ; Fässler, R (AMER SOC MICROBIOLOGY, 1999-11)
    Matrilin 1, or cartilage matrix protein, is a member of a novel family of extracellular matrix proteins. To date, four members of the family have been identified, but their biological role is unknown. Matrilin 1 and matrilin 3 are expressed in cartilage, while matrilin 2 and matrilin 4 are present in many tissues. Here we describe the generation and analysis of mice carrying a null mutation in the Crtm gene encoding matrilin 1. Anatomical and histological studies demonstrated normal development of homozygous mutant mice. Northern blot and biochemical analyses show no compensatory up-regulation of matrilin 2 or 3 in the cartilage of knockout mice. Although matrilin 1 interacts with the collagen II and aggrecan networks of cartilage, suggesting that it may play a role in cartilage tissue organization, studies of collagen extractability indicated that collagen fibril maturation and covalent cross-linking were unaffected by the absence of matrilin 1. Ultrastructural analysis did not reveal any abnormalities of matrix organization. These data suggest that matrilin 1 is not critically required for cartilage structure and function and that matrilin 1 and matrilin 3 may have functionally redundant roles.
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    Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex I
    Lazarou, M ; McKenzie, M ; Ohtake, A ; Thorburn, DR ; Ryan, MT (AMER SOC MICROBIOLOGY, 2007-06)
    Complex I of the respiratory chain is composed of at least 45 subunits that assemble together at the mitochondrial inner membrane. Defects in human complex I result in energy generation disorders and are also implicated in Parkinson's disease and altered apoptotic signaling. The assembly of this complex is poorly understood and is complicated by its large size and its regulation by two genomes, with seven subunits encoded by mitochondrial DNA (mtDNA) and the remainder encoded by nuclear genes. Here we analyzed the assembly of a number of mtDNA- and nuclear-gene-encoded subunits into complex I. We found that mtDNA-encoded subunits first assemble into intermediate complexes and require significant chase times for their integration into the holoenzyme. In contrast, a set of newly imported nuclear-gene-encoded subunits integrate with preexisting complex I subunits to form intermediates and/or the fully assembly holoenzyme. One of the intermediate complexes represents a subassembly associated with the chaperone B17.2L. By using isolated patient mitochondria, we show that this subassembly is a productive intermediate in complex I assembly since import of the missing subunit restores complex I assembly. Our studies point to a mechanism of complex I biogenesis involving two complementary processes, (i) synthesis of mtDNA-encoded subunits to seed de novo assembly and (ii) exchange of preexisting subunits with newly imported ones to maintain complex I homeostasis. Subunit exchange may also act as an efficient mechanism to prevent the accumulation of oxidatively damaged subunits that would otherwise be detrimental to mitochondrial oxidative phosphorylation and have the potential to cause disease.
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    ANTIGENIC VARIATION IN PLASMODIUM-FALCIPARUM
    BIGGS, BA ; GOOZE, L ; WYCHERLEY, K ; WOLLISH, W ; SOUTHWELL, B ; LEECH, JH ; BROWN, GV (NATL ACAD SCIENCES, 1991-10)
    Antigenic variation of infectious organisms is a major factor in evasion of the host immune response. However, there has been no definitive demonstration of this phenomenon in the malaria parasite Plasmodium falciparum. In this study, cloned parasites were examined serologically and biochemically for the expression of erythrocyte surface antigens. A cloned line of P. falciparum gave rise to progeny that expressed antigenically distinct forms of an erythrocyte surface antigen but were otherwise identical. This demonstrates that antigenic differences on the surface of P. falciparum-infected erythrocytes can arise by antigenic variation of clonal parasite populations. The antigenic differences were shown to result from antigenic variation of the parasite-encoded protein, the P. falciparum erythrocyte membrane protein 1.
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    The Australasian Society for Infectious Diseases guidelines for the diagnosis, management and prevention of infections in recently arrived refugees: an abridged outline
    Murray, RJ ; Davis, JS ; Burgner, DP (WILEY, 2009-04-20)
    About 13,000 refugees are currently accepted for migration into Australia each year, many of whom have spent protracted periods living in extremely disadvantaged circumstances. As a result, medical practitioners are increasingly managing recently arrived refugees with acute and chronic infectious diseases. The Australasian Society for Infectious Diseases has formulated guidelines for the diagnosis, management and prevention of infection in newly arrived refugees. This article is an abridged version of the guidelines, which are available in full at . All refugees should be offered a comprehensive health assessment, ideally within 1 month of arrival in Australia, that includes screening for and treatment of tuberculosis, malaria, blood-borne viral infections, schistosomiasis, helminth infection, sexually transmitted infections, and other infections (eg, Helicobacter pylori) as indicated by clinical assessment; and assessment of immunisation status, and catch-up immunisations where appropriate. The assessment can be undertaken by a general practitioner or within a multidisciplinary refugee health clinic, with use of an appropriate interpreter when required. The initial assessment should take place over at least two visits: the first for initial assessment and investigation and the second for review of results and treatment or referral.
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    Avoiding conflict: What do adolescents with disordered eating say about their mothers in music therapy?
    McFerran, K ; Baker, F ; Kildea, C ; Patton, G ; Sawyer, S (SAGE Publications, 2008-06-01)
    Music therapy is an integral part of the inpatient treatment programme for young women with disordered eating at the Royal Children's Hospital in Melbourne. As part of ongoing clinical audit activities, an investigation was undertaken to analyse retrospectively the lyrics of young women who had participated in the music therapy programme. The¬¬¬¬¬¬ purpose was to monitor and improve local clinical practice and clarify the specific contribution of music therapy to the diagnosis of anorexia nervosa. Results highlighted the role of mothers in the experiences of the young participants, with references to this relationship exceeding those to any other relationships. These findings are discussed in conjunction with an abandoned study where parental consent was not forthcoming for participation in a group music therapy research project. This article promotes a continuing awareness of the importance of the mother-daughter relationship in the treatment of eating disorders.
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    New Mid-Cretaceous (latest Albian) dinosaurs fromWinton, Queensland, Australia.
    Hocknull, SA ; White, MA ; Tischler, TR ; Cook, AG ; Calleja, ND ; Sloan, T ; Elliott, DA ; Sereno, P (Public Library of Science (PLoS), 2009-07-03)
    BACKGROUND: Australia's dinosaurian fossil record is exceptionally poor compared to that of other similar-sized continents. Most taxa are known from fragmentary isolated remains with uncertain taxonomic and phylogenetic placement. A better understanding of the Australian dinosaurian record is crucial to understanding the global palaeobiogeography of dinosaurian groups, including groups previously considered to have had Gondwanan origins, such as the titanosaurs and carcharodontosaurids. METHODOLOGY/PRINCIPAL FINDINGS: We describe three new dinosaurs from the late Early Cretaceous (latest Albian) Winton Formation of eastern Australia, including; Wintonotitan wattsi gen. et sp. nov., a basal titanosauriform; Diamantinasaurus matildae gen. et sp. nov., a derived lithostrotian titanosaur; and Australovenator wintonensis gen. et sp. nov., an allosauroid. We compare an isolated astragalus from the Early Cretaceous of southern Australia; formerly identified as Allosaurus sp., and conclude that it most-likely represents Australovenator sp. CONCLUSION/SIGNIFICANCE: The occurrence of Australovenator from the Aptian to latest Albian confirms the presence in Australia of allosauroids basal to the Carcharodontosauridae. These new taxa, along with the fragmentary remains of other taxa, indicate a diverse Early Cretaceous sauropod and theropod fauna in Australia, including plesiomorphic forms (e.g. Wintonotitan and Australovenator) and more derived forms (e.g. Diamantinasaurus).
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    Decline in Physical Fitness From Childhood to Adulthood Associated With Increased Obesity and Insulin Resistance in Adults
    Dwyer, T ; Magnussen, CG ; Schmidt, MD ; Ukoumunne, OC ; Ponsonby, A-L ; Raitakari, OT ; Zimmet, PZ ; Blair, SN ; Thomson, R ; Cleland, VJ ; Venn, A (AMER DIABETES ASSOC, 2009-04)
    OBJECTIVE: To examine how fitness in both childhood and adulthood is associated with adult obesity and insulin resistance. RESEARCH DESIGN AND METHODS: A prospective cohort study set in Australia in 2004-2006 followed up a cohort of 647 adults who had participated in the Australian Schools Health and Fitness Survey in 1985 and who had undergone anthropometry and cardiorespiratory fitness assessment during the survey. Outcome measures were insulin resistance and obesity, defined as a homeostasis model assessment index above the 75th sex-specific percentile and BMI >or=30 kg/m(2), respectively. RESULTS: Lower levels of child cardiorespiratory fitness were associated with increased odds of adult obesity (adjusted odds ratio [OR] per unit decrease 3.0 [95% CI 1.6-5.6]) and insulin resistance (1.7 [1.1-2.6]). A decline in fitness level between childhood and adulthood was associated with increased obesity (4.5 [2.6-7.7]) and insulin resistance (2.1 [1.5-2.9]) per unit decline. CONCLUSIONS: A decline in fitness from childhood to adulthood, and by inference a decline in physical activity, is associated with obesity and insulin resistance in adulthood. Programs aimed at maintaining high childhood physical activity levels into adulthood may have potential for reducing the burden of obesity and type 2 diabetes in adults.
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    Duration of Nocturnal Hypoglycemia Before Seizures
    Buckingham, B ; Wilson, DM ; Lecher, T ; Hanas, R ; Kaiserman, K ; Cameron, F (AMER DIABETES ASSOC, 2008-11)
    OBJECTIVE: Despite a high incidence of nocturnal hypoglycemia documented by the use of continuous glucose monitoring (CGM), there are no reports in the literature of nocturnal hypoglycemic seizures while a patient is wearing a CGM device. RESEARCH DESIGN AND METHODS: In this article, we describe four such cases and assess the duration of nocturnal hypoglycemia before the seizure. RESULTS: In the cases where patients had a nocturnal hypoglycemic seizure while wearing a CGM device, sensor hypoglycemia (<60 mg/dl) was documented on the CGM record for 2.25-4 h before seizure activity. CONCLUSIONS: Even with a subcutaneous glucose lag of 18 min when compared with blood glucose measurements, glucose sensors have time to provide clinically meaningful alarms. Current nocturnal hypoglycemic alarms need to be improved, however, since patients can sleep through the current alarm systems.
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    Paradoxical myopic shift following cycloplegia in retinopathy of prematurity patients: a case series.
    London, NJ ; Carden, SM ; Good, WV (Springer Science and Business Media LLC, 2009-08-25)
    INTRODUCTION: Spectacle non-compliance is a significant problem in pediatric patients, and may have a variety of consequences. Non-compliance with myopic refractive correction could be secondary to a variety of issues, including age, discomfort, gender, urban vs. rural residence, presenting visual acuity, and degree of refractive error. We observed a phenomenon in our pediatric patients with retinopathy of prematurity that may add another possible explanation: incorrect prescription due to measures of increased, rather than decreased, myopia after cycloplegia. CASE PRESENTATION: An unmasked, prospective study of 8 consecutive patients seen in a single practice. Retinoscopic refraction measurements were obtained before and after pharmacologic cycloplegia. In all 13 eyes, there was either no change (2 eyes) or a myopic shift (11 eyes) in the measured refractive error. The average change in refraction was -1.58 and -1.54 for the right and left eyes, respectively (range 0 to -3.00 OD and 0 to -3.00 OS). CONCLUSIONS: The contribution of ocular components to refractive status differs between ROP and non-ROP eyes. Unanticipated myopic shift following cycloplegia in ROP patients may result in inappropriate glasses prescription with poor correction of visual acuity. This may contribute to spectacle noncompliance in this group.